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Primary cilia

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https://www.readbyqxmd.com/read/28922052/applications-of-emerging-transmission-electron-microscopy-technology-in-pcd-research-and-diagnosis
#1
Amelia Shoemark
Primary Ciliary Dyskinesia (PCD) is a heterogeneous genetic condition characterized by dysfunction of motile cilia. Patients suffer from chronic infection and inflammation of the upper and lower respiratory tract. Diagnosis of PCD is confirmed by identification of a hallmark defect of ciliary ultrastructure or by identification of biallelic pathogenic mutations in a known PCD gene. Since the first description of PCD in 1976, assessment of ciliary ultrastructure by transmission electron microscopy (TEM) has been central to diagnosis and research...
September 18, 2017: Ultrastructural Pathology
https://www.readbyqxmd.com/read/28915070/value-of-transmission-electron-microscopy-for-primary-ciliary-dyskinesia-diagnosis-in-the-era-of-molecular-medicine-genetic-defects-with-normal-and-non-diagnostic-ciliary-ultrastructure
#2
Adam J Shapiro, Margaret W Leigh
Primary ciliary dyskinesia (PCD) is a genetic disorder causing chronic oto-sino-pulmonary disease. No single diagnostic test will detect all PCD cases. Transmission electron microscopy (TEM) of respiratory cilia was previously considered the gold standard diagnostic test for PCD, but 30% of all PCD cases have either normal ciliary ultrastructure or subtle changes which are non-diagnostic. These cases are identified through alternate diagnostic tests, including nasal nitric oxide measurement, high-speed videomicroscopy analysis, immunofluorescent staining of axonemal proteins, and/or mutation analysis of various PCD causing genes...
September 15, 2017: Ultrastructural Pathology
https://www.readbyqxmd.com/read/28913352/new-roles-of-the-primary-cilium-in-autophagy
#3
REVIEW
Yenniffer Ávalos, Daniel Peña-Oyarzun, Mauricio Budini, Eugenia Morselli, Alfredo Criollo
The primary cilium is a nonmotile organelle that emanates from the surface of multiple cell types and receives signals from the environment to regulate intracellular signaling pathways. The presence of cilia, as well as their length, is important for proper cell function; shortened, elongated, or absent cilia are associated with pathological conditions. Interestingly, it has recently been shown that the molecular machinery involved in autophagy, the process of recycling of intracellular material to maintain cellular and tissue homeostasis, participates in ciliogenesis...
2017: BioMed Research International
https://www.readbyqxmd.com/read/28912507/fast-track-development-of-an-in-vitro-3d-lung-immune-cell-model-to-study-aspergillus-infections
#4
P Chandorkar, W Posch, V Zaderer, M Blatzer, M Steger, C G Ammann, U Binder, M Hermann, P Hörtnagl, C Lass-Flörl, D Wilflingseder
To study interactions of airborne pathogens, e.g. Aspergillus (A.) fumigatus with upper and lower respiratory tract epithelial and immune cells, we set up a perfused 3D human bronchial and small airway epithelial cell system. Culturing of normal human bronchial or small airway epithelial (NHBE, SAE) cells under air liquid interphase (ALI) and perfusion resulted in a significantly accelerated development of the lung epithelia associated with higher ciliogenesis, cilia movement, mucus-production and improved barrier function compared to growth under static conditions...
September 14, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28901443/basic-fibroblast-growth-factor-increases-ift88-expression-in-chondrocytes
#5
Daolu Zhan, Wei Xiang, Fengjing Guo, Yuanzheng Ma
Intraflagellar transport protein 88 (IFT88) is protein crucial for the assembly and maintenance of primary cilia in chondrocytes. Primary cilia regulate mechanical and chemical signals in chondrocytes; however, the effects of cytokines on IFT88 expression and cilia formation and maintenance remain to be elucidated. Therefore, the role of basic fibroblast growth factor (bFGF) on IFT88 expression were examined in theATDC5 murine chondrocytic line, in order to investigate the signaling pathways involved in this process...
September 8, 2017: Molecular Medicine Reports
https://www.readbyqxmd.com/read/28891733/application-of-laboratory-and-digital-techniques-for-visual-enhancement-during-the-ultrastructural-assessment-of-cilia
#6
Josef A Schroeder
Routine diagnostic electron microscopy of primary ciliary dyskinesia (PCD) is based on the findings of ultrastructural defects of axonemal components. Assessment of the typical abnormalities can be enhanced by improving the sample preservation status using tannic acid (TA) as additive in the biopsy fixation or processing steps. Another option is the implementation of computer-assisted image analysis tools. Advancements in high-resolution 3D visualization of the axonemal structure have been noted, with great potential for the future diagnosis of inherited cilia disorders...
September 11, 2017: Ultrastructural Pathology
https://www.readbyqxmd.com/read/28886341/cdk10-mutations-in-humans-and-mice-cause-severe-growth-retardation-spine-malformations-and-developmental-delays
#7
Christian Windpassinger, Juliette Piard, Carine Bonnard, Majid Alfadhel, Shuhui Lim, Xavier Bisteau, Stéphane Blouin, Nur'Ain B Ali, Alvin Yu Jin Ng, Hao Lu, Sumanty Tohari, S Zakiah A Talib, Noémi van Hul, Matias J Caldez, Lionel Van Maldergem, Gökhan Yigit, Hülya Kayserili, Sameh A Youssef, Vincenzo Coppola, Alain de Bruin, Lino Tessarollo, Hyungwon Choi, Verena Rupp, Katharina Roetzer, Paul Roschger, Klaus Klaushofer, Janine Altmüller, Sudipto Roy, Byrappa Venkatesh, Rudolf Ganger, Franz Grill, Farid Ben Chehida, Bernd Wollnik, Umut Altunoglu, Ali Al Kaissi, Bruno Reversade, Philipp Kaldis
In five separate families, we identified nine individuals affected by a previously unidentified syndrome characterized by growth retardation, spine malformation, facial dysmorphisms, and developmental delays. Using homozygosity mapping, array CGH, and exome sequencing, we uncovered bi-allelic loss-of-function CDK10 mutations segregating with this disease. CDK10 is a protein kinase that partners with cyclin M to phosphorylate substrates such as ETS2 and PKN2 in order to modulate cellular growth. To validate and model the pathogenicity of these CDK10 germline mutations, we generated conditional-knockout mice...
September 7, 2017: American Journal of Human Genetics
https://www.readbyqxmd.com/read/28883651/the-role-of-transcriptional-factor-p63-in-regulation-of-epithelial-barrier-and-ciliogenesis-of-human-nasal-epithelial-cells
#8
Yakuto Kaneko, Takayuki Kohno, Takuya Kakuki, Ken-Ichi Takano, Noriko Ogasawara, Ryo Miyata, Shin Kikuchi, Takumi Konno, Tsuyoshi Ohkuni, Ryoto Yajima, Akito Kakiuchi, Shin-Ichi Yokota, Tetsuo Himi, Takashi Kojima
Disruption of nasal epithelial tight junctions (TJs) and ciliary dysfunction are found in patients with chronic rhinosinusitis (CRS) and nasal polyps (NPs), along with an increase of p63-positive basal cells and histone deacetylase (HDAC) activity. To investigate these mechanisms, primary cultures of HNECs transfected with human telomerase reverse transcriptase (hTERT-HNECs) were transfected with siRNAs of TAp63 and ΔNp63, treated with the NF-kB inhibitor curucumin and inhibitors of HDACs, and infected with respiratory syncytial virus (RSV)...
September 7, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28877972/clinical-care-for-primary-ciliary-dyskinesia-current-challenges-and-future-directions
#9
REVIEW
Bruna Rubbo, Jane S Lucas
Primary ciliary dyskinesia (PCD) is a rare genetic disease that affects the motility of cilia, leading to impaired mucociliary clearance. It is estimated that the vast majority of patients with PCD have not been diagnosed as such, providing a major obstacle to delivering appropriate care. Challenges in diagnosing PCD include lack of disease-specific symptoms and absence of a single, "gold standard", diagnostic test. Management of patients is currently not based on high-level evidence because research findings are mostly derived from small observational studies with limited follow-up period...
September 30, 2017: European Respiratory Review: An Official Journal of the European Respiratory Society
https://www.readbyqxmd.com/read/28877752/spen-a-new-player-in-primary-cilia-formation-and-cell-migration-in-breast-cancer
#10
Stéphanie Légaré, Catherine Chabot, Mark Basik
BACKGROUND: The primary cilium is a microtubule-based and nonmotile organelle functioning as a cellular antenna that is involved in the regulation of cell proliferation, differentiation, and migration. In breast cancer cells, the primary cilium is a structure that decreases in incidence with increasing degrees of transformation and may be biologically more important in estrogen receptor (ERα)-negative breast cancer cells. Split ends (SPEN) is an ERα corepressor that we have identified as a tumor suppressor protein in ERα-positive breast cancer cells whose hormone-independent roles in breast cancer have never been explored...
September 6, 2017: Breast Cancer Research: BCR
https://www.readbyqxmd.com/read/28871046/loss-of-ocrl-increases-ciliary-pi-4-5-p2-in-oculocerebrorenal-syndrome-of-lowe
#11
Philipp P Prosseda, Na Luo, Biao Wang, Jorge A Alvarado, Yang Hu, Yang Sun
Lowe syndrome is a rare X-linked disorder characterized by bilateral congenital cataracts and glaucoma, mental retardation, and proximal renal tubular dysfunction. Mutations in OCRL1, an inositol polyphosphate 5-phosphatase that dephosphorylates PI(4,5)P2, cause Lowe syndrome. Previously we showed that OCRL localizes to the primary cilium, which has a distinct membrane phospholipid composition, but disruption of phosphoinositides in the ciliary membrane poorly understood. Here we demonstrate that cilia from Lowe syndrome patient fibroblasts exhibit increased levels of PI(4,5)P2 and decreased levels of PI4P...
September 4, 2017: Journal of Cell Science
https://www.readbyqxmd.com/read/28863455/characterization-of-2-novel-ependymoma-cell-lines-with-chromosome-1q-gain-derived-from-posterior-fossa-tumors-of-childhood
#12
Vladimir Amani, Andrew M Donson, Seth C Lummus, Eric W Prince, Andrea M Griesinger, Davis A Witt, Todd C Hankinson, Michael H Handler, Kathleen Dorris, Rajeev Vibhakar, Nicholas K Foreman, Lindsey M Hoffman
Ependymoma (EPN) is a common brain tumor of childhood that, despite standard surgery and radiation therapy, has a relapse rate of 50%. Clinical trials have been unsuccessful in improving outcome by addition of chemotherapy, and identification of novel therapeutics has been hampered by a lack of in vitro and in vivo models. We describe 2 unique EPN cell lines (811 and 928) derived from recurrent intracranial metastases. Both cell lines harbor the high-risk chromosome 1q gain (1q+) and a derivative chromosome 6, and both are classified as molecular group A according to transcriptomic analysis...
July 1, 2017: Journal of Neuropathology and Experimental Neurology
https://www.readbyqxmd.com/read/28855375/shear-stress-induces-non-canonical-autophagic-flux-in-intestinal-epithelial-monolayers
#13
Sun Wook Kim, Jonathan Ehrman, Mok-Ryeon Ahn, Jumpei Kondo, Andrea A Mancheno Lopez, Yun Sik Oh, Hanjune X Kim, Scott W Crawley, James R Goldenring, Matthew J Tyska, Erin C Rericha, Ken S Lau
Flow of fluids through the gut, such as milk from a neonatal diet, generates a shear stress on the unilaminar epithelium lining the lumen.  We report that exposure to physiological levels of fluid shear stress leads to the formation of large vacuoles, containing extracellular content, within polarizing intestinal epithelial cell monolayers.  These observations lead to two questions: how can cells lacking primary cilia transduce shear stress, and what molecular pathways support the formation of vacuoles that can exceed 80% of the cell volume...
August 30, 2017: Molecular Biology of the Cell
https://www.readbyqxmd.com/read/28843978/primary-cilia-cell-and-molecular-mechanosensors-directing-whole-tissue-function
#14
REVIEW
Milos Spasic, Christopher R Jacobs
Primary cilia are immotile, microtubule-based organelles extending from the surface of nearly every mammalian cell. Mechanical stimulation causes deflection of the primary cilium, initiating downstream signaling cascades to the rest of the cell. The cilium forms a unique subcellular microdomain, and defects in ciliary protein composition or physical structure have been associated with a myriad of human pathologies. In this review, we discuss the importance of ciliary mechanotransduction at the cell and tissue level, and how furthering our molecular understanding of primary cilia mechanobiology may lead to therapeutic strategies to treat human diseases...
August 23, 2017: Seminars in Cell & Developmental Biology
https://www.readbyqxmd.com/read/28842217/cytoskeleton-related-regulation-of-primary-cilia-shortening-mediated-by-melanin-concentrating-hormone-receptor-1
#15
Sakura Tomoshige, Yuki Kobayashi, Kosuke Hosoba, Akie Hamamoto, Tatsuo Miyamoto, Yumiko Saito
Primary cilia are specialized microtubule-based organelles. Their importance is highlighted by the gamut of ciliary diseases associated with various syndromes including diabetes and obesity. Primary cilia serve as signaling hubs through selective interactions with ion channels and conventional G-protein-coupled receptors (GPCRs). Melanin-concentrating hormone (MCH) receptor 1 (MCHR1), a key regulator of feeding, is selectively expressed in neuronal primary cilia in distinct regions of the mouse brain. We previously found that MCH acts on ciliary MCHR1 and induces cilia shortening through a Gi/o-dependent Akt pathway with no cell cycle progression...
August 22, 2017: General and Comparative Endocrinology
https://www.readbyqxmd.com/read/28842124/phencyclidine-induced-dysregulation-of-primary-cilia-in-the-rodent-brain
#16
Hiroki Shiwaku, Asami Umino, Masakazu Umino, Toru Nishikawa
Significant roles of the primary cilia in the central nervous system have been reported in neural generation and cognitive functions. However, little is known about the possible pathological changes in brain primary cilia in neuropsychiatric disorders. To obtain an insight into the relationship between cilial dysregulation and schizophrenia, we presently investigated the effects of psychotomimetics, phencyclidine, MK-801 (dizocilpine), and methamphetamine, on morphological and molecular indices in the rodent brain...
August 22, 2017: Brain Research
https://www.readbyqxmd.com/read/28827763/peroxiredoxin1-a-novel-regulator-of-pronephros-development-influences-retinoic-acid-and-wnt-signaling-by-controlling-ros-levels
#17
Soomin Chae, Hyun-Kyung Lee, Yoo-Kyung Kim, Hyo Jung Sim, Yoorim Ji, Chowon Kim, Tayaba Ismail, Jeen-Woo Park, Oh-Shin Kwon, Beom-Sik Kang, Dong-Seok Lee, Jong-Sup Bae, Sang-Hyun Kim, Kyoung-Jin Min, Taeg Kyu Kwon, Mae-Ja Park, Jin-Kwan Han, Taejoon Kwon, Tae-Joo Park, Hyun-Shik Lee
Peroxiredoxin1 (Prdx1) is an antioxidant enzyme belonging to the peroxiredoxin family of proteins. Prdx1 catalyzes the reduction of H2O2 and alkyl hydroperoxide and plays an important role in different biological processes. Prdx1 also participates in various age-related diseases and cancers. In this study, we investigated the role of Prdx1 in pronephros development during embryogenesis. Prdx1 knockdown markedly inhibited proximal tubule formation in the pronephros and significantly increased the cellular levels of reactive oxygen species (ROS), which impaired primary cilia formation...
August 21, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28817564/cell-cycle-related-kinase-ccrk-regulates-ciliogenesis-and-hedgehog-signaling-in-mice
#18
Ashley Snouffer, Desmond Brown, Hankyu Lee, Jonathon Walsh, Floria Lupu, Ryan Norman, Karl Lechtreck, Hyuk Wan Ko, Jonathan Eggenschwiler
The Hedgehog (Hh) signaling pathway plays a key role in cell fate specification, proliferation, and survival during mammalian development. Cells require a small organelle, the primary cilium, to respond properly to Hh signals and the key regulators of Hh signal transduction exhibit dynamic localization to this organelle when the pathway is activated. Here, we investigate the role of Cell Cycle Related kinase (CCRK) in regulation of cilium-dependent Hh signaling in the mouse. Mice mutant for Ccrk exhibit a variety of developmental defects indicative of inappropriate regulation of this pathway...
August 2017: PLoS Genetics
https://www.readbyqxmd.com/read/28811631/development-of-a-primary-human-co-culture-model-of-inflamed-airway-mucosa
#19
Lael M Yonker, Hongmei Mou, Kengyeh K Chu, Michael A Pazos, Huimin Leung, Dongyao Cui, Jinhyeob Ryu, Rhianna M Hibbler, Alexander D Eaton, Tim N Ford, J R Falck, T Bernard Kinane, Guillermo J Tearney, Jayaraj Rajagopal, Bryan P Hurley
Neutrophil breach of the mucosal surface is a common pathological consequence of infection. We present an advanced co-culture model to explore neutrophil transepithelial migration utilizing airway mucosal barriers differentiated from primary human airway basal cells and examined by advanced imaging. Human airway basal cells were differentiated and cultured at air-liquid interface (ALI) on the underside of 3 µm pore-sized transwells, compatible with the study of transmigrating neutrophils. Inverted ALIs exhibit beating cilia and mucus production, consistent with conventional ALIs, as visualized by micro-optical coherence tomography (µOCT)...
August 15, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28811097/type-i-collagen-promotes-primary-cilia-growth-through-down-regulating-hdac6-mediated-autophagy-in-confluent-mouse-embryo-fibroblast-3t3-l1-cells
#20
Qian Xu, Weiwei Liu, Xiaoling Liu, Wuxiyar Otkur, Toshihiko Hayashi, Masayuki Yamato, Hitomi Fujisaki, Shunji Hattori, Shin-Ichi Tashiro, Takashi Ikejima
Primary cilia are microtubule-based organelles that extend from nearly all vertebrate cells. Abnormal ciliogenesis and cilia length are suggested to be associated with hypertension and obesity as well as diseases such as Meckel-Gruber syndrome. Extracellular matrix (ECM), comprising cellular microenvironment, influences cell shape and proliferation. However, influence of ECM on cilia biogenesis has not been well studied. In this study we examined the effects of type I collagen (col I), the major component of ECM, on primary cilia growth...
August 12, 2017: Journal of Bioscience and Bioengineering
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